Pemphigus Herpetiformis Treatment & Management

Updated: Nov 16, 2018
  • Author: Blanca Anais Estupiñan; Chief Editor: William D James, MD  more...
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Treatment

Medical Care

Pemphigus herpetiformis is responsive to anti-inflammatory and immunosuppressive medications. Dapsone is the drug of choice if the patient tolerates the treatment. Among its many anti-inflammatory actions, dapsone has been shown to decrease neutrophil migration, which may lessen the immune response seen in pemphigus herpetiformis. [34]

In severe cases of pemphigus herpetiformis that do not respond to conventional treatments (systemic corticosteroid and immunosuppressives), also consider using a monoclonal antibody anti-CD20 (rituximab), which has specific actions against B lymphocytes. [35] Rituximab has shown very good clinical efficacy for patients with pemphigus vulgaris, a form of pemphigus that is usually more severe than pemphigus herpetiformis. One report noted success with the combination of rituximab and intravenous immune globulin. [36]

The disease activities of pemphigus herpetiformis rarely require hospitalization. In a few patients, short-term hospitalization (< 10 d) may be helpful.

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Activity

No specific activity restrictions are indicated for pemphigus herpetiformis patients; however, advise patients to avoid injury during the active disease period.

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Prevention

For pemphigus herpetiformis patients who are treated with systemic corticosteroid for longer than 1 month, combined calcium and vitamin D supplements should be instituted to prevent osteoporosis. The dosage and frequency of these supplements are stated in the 1996 recommendations established by the American College of Rheumatology Task Force. [37]

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Long-Term Monitoring

The disease activities of pemphigus herpetiformis range from mild to moderate. During the active disease period, provide follow-up care for patients on a monthly outpatient basis. These visits ensure that (1) patients are responding clinically to prescribed medications and (2) patients experience no serious adverse effects that require a change of medications. Typically, follow-up visits are not needed for patients in clinical remission and not on treatment.

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